Carney's syndrome: complex myxomas. Report of four cases and review of the literature.

Cardiac myxomas are rare tumors. They usually appear as a sporadic isolated condition in the left atrium of middle-aged women with no other coincidental pathology. Carney and others have described in young people a special complex group of cardiac myxomas associated to a distinctive complex pathology, giving identity to the "Syndrome Myxoma" or "Carney's Syndrome". Four additional cases of this syndrome, treated from 1977 to 1999 at the Hospital Clínico de la Universidad de Chile are presented here with a comprehensive review of the literature, accumulating 100 cases. The main features of our cases include the presence of malignant non cardiac tumors, a familial trend, follow-up of 23 years and an iterative recurrence in the elder case. To date all patients are tumor free. Reviewing the literature, patients with Carney's Syndrome were younger, with a mean age of 26 years and female predominance (62%). Cardiac myxomas affected the four chambers of the heart: 64% the left atrium; 44% the right atrium; 14% the left ventricle and 12% the right ventricle. They were multiple tumors in 41% and involved more than one chamber in 31%, being synchronous or metachronous. There was a marked familial trend (52%), a high incidence of recurrence (20%), with more than one occurring in half the cases. Extra-cardiac involvement consisted of: 68% pigmented skin lesions, 40% cutaneous myxomas, 37% adrenal cortical disease, 27% myxoid mammary fibroadenoma and 34% male patients with testes tumors. A low percentage had pituitary adenoma, melanotic schwannomas and thyroid disease. The diagnosis is made when two or more of these criteria are present. In agreement with these findings the four chambers of the heart should be examined at surgery for atypical myxoma locations, right atriotomy and combined superior-transseptal approach improve exposure of the cavities, careful screening of the first degree family members should be conducted, and closed short and long term follow up controls are important. Complex myxoma appears as a multi-systemic disorder, occasionally having an ominous prognosis and malignant potentiality, and is still undergoing investigation for better understanding and identification.

Miomatósis uterina. Estudio retrospectivo 1993

El presente es un estudio retrospectivo realizado amediante la revisión de historias clínicas con diagnóstico clínico e hispatológico de miomatósis uterina, desde enero a diciembre de 1993, en el Hospital Gineco-obstétrico Isidro Ayora. Obtuvimos un total de 60 casos, la media de la edad de las pacientes fue de 42.96 años con una desviación estandar de 6.18. El 90.3 por ciento fueron multíparas y solo un 4.8 por ciento utilizaron anticonceptivos orales,. El cuadro clínico dominante fue: dolor hipogástrico, sangrado genital anormal, palpitación dolorosa, irregularidad uterina y masa pélvica. Se confirmó el diagnostico mediante ECO pélvico en 96.6 por ciento de pacientes. El 63.3 por ciento presentó PAP TEST clase II con una media de la edad en estas pacientes de 43.25 años. En la mayor parte de pacientes el tumor invadió una sola capa uterina (45 por ciento) y de estos el 74 por ciento fue de localizaci{on intramural...